1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
b) Generalized junctional epidermolysis bullosa, non-Herlitz subtype (JEB-nH)
c) EB simplex, Dowling-Meara subtype
d) Dominant dystrophic EB
e) Severe generalized recessive dystrophic EB (RDEB, severe generalized; formerly named RDEB, Hallopeau-Siemens)
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
a) Type IV collagen
b) Bullous pemphigoid antigen 1 (230-kD)
c) Laminin-332
d) Type VII collagen
e) Transglutaminase
To learn the answers, go to page 2
{{pagebreak}}
BOARD REVIEW ANSWERS:
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
This child has symmetrically arrayed, periorificial, exuberant granulation tissue, a finding that is virtually pathognomonic of the Herlitz subtype of generalized JEB. Other skin sites affected by such a finding include the nape of the neck, lumbosacral region, and periungual folds. The nares and upper airway may also harbor granulation tissue like this, and may markedly obstruct air flow. JEB-H is an autosomal recessive disorder resulting from mutations arising in the genes encoding for any of the 3 subunits of laminin-332 (formerly named laminin-5), a 3-chained, cross-configured macromolecule which spans the laimina lucida of the dermoepidermal junction, attaching epidermis to dermis.
The classification system for inherited EB was revised in 2007 and published in the Journal of the American Academy of Dermatology in 2008. A significantly revised version incorporating several new subtypes characterized by novel phenotypes or newly discovered genes was published in the Journal of the American Academy of Dermatology in 2014. The key features of the revised classification system include that inherited EB has been expanded into 4 major groups, to now include Kindler syndrome; EB simplex has been subdivided into 2 major subgroups (basal and suprabasal); several of the traditional subtypes with eponyms are now re-named; additional subtypes have been added to include the laryngo-onycho-cutaneous syndrome (LOC syndrome; Shabbir syndrome); additional modes of transmission have been noted; and detailed summaries are available which describe transmission electron microscopy findings, abnormalities in basement membrane antigen expression, the most common types of gene mutations within each subtype, and comparative clinical findings.
References
Fine JD, Bauer EA, McGuire J, Moshell A, eds. Epidermolysis Bullosa: Clinical, Epidemiologic, and Laboratory Advances, and the Findings of the National Epidermolysis Bullosa Registry. Baltimore MD: Johns Hopkins University Press; 1999:520.
Fine JD, Hintner H, eds. Life with Epidermolysis Bullosa. Etiology, Diagnosis, Multidisciplinary Care and Therapy. New York NY: Springer; 2009:338.
Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol. 2008;58(6):931-950.
Fine JD, Bruckner-Tuderman L, Eady RA, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014;70(6):1003-11026.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367-384; quiz 385-386.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. J Am Acad Dermatol. 2009;61(3):387-402; quiz 403-404.
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
d) Type IV collagen
The tissue-bound autoantibodies directly beneath the lamina densa, co-localizing with the anchoring fibrils is depicted in this direct immunoelectron micrograph (Figure). The patient has either epidermolysis bullosa acquisita or the bullous eruption of systemic lupus erythematosus. Note: the faint gray linear band visible in the region of the dermoepidermal junction is the lamina densa “B”, which is devoid of overlying autoantibodies. The latter (“D”) are present solely within the sublamina densa zone. The only other autoimmune bullous disease which may have immunoglobulin deposits in linear array within the uppermost papillary dermis is linear IgA dermatosis (LAD), although most patients with LAD have their deposits within the lamina lucida; it is as yet unknown what is the antigenic target of these LAD-associated sublamina densa immunoreactants.
References
Gammon WR, Briggaman RA. Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Dermatol Clin. 1993;11(3):535-547.
Remington J, Chen M, Burnett J, Woodley DT. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Curr Dir Autoimmun. 2008;10:195-205.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
b) Generalized junctional epidermolysis bullosa, non-Herlitz subtype (JEB-nH)
c) EB simplex, Dowling-Meara subtype
d) Dominant dystrophic EB
e) Severe generalized recessive dystrophic EB (RDEB, severe generalized; formerly named RDEB, Hallopeau-Siemens)
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
a) Type IV collagen
b) Bullous pemphigoid antigen 1 (230-kD)
c) Laminin-332
d) Type VII collagen
e) Transglutaminase
BOARD REVIEW ANSWERS:
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
This child has symmetrically arrayed, periorificial, exuberant granulation tissue, a finding that is virtually pathognomonic of the Herlitz subtype of generalized JEB. Other skin sites affected by such a finding include the nape of the neck, lumbosacral region, and periungual folds. The nares and upper airway may also harbor granulation tissue like this, and may markedly obstruct air flow. JEB-H is an autosomal recessive disorder resulting from mutations arising in the genes encoding for any of the 3 subunits of laminin-332 (formerly named laminin-5), a 3-chained, cross-configured macromolecule which spans the laimina lucida of the dermoepidermal junction, attaching epidermis to dermis.
The classification system for inherited EB was revised in 2007 and published in the Journal of the American Academy of Dermatology in 2008. A significantly revised version incorporating several new subtypes characterized by novel phenotypes or newly discovered genes was published in the Journal of the American Academy of Dermatology in 2014. The key features of the revised classification system include that inherited EB has been expanded into 4 major groups, to now include Kindler syndrome; EB simplex has been subdivided into 2 major subgroups (basal and suprabasal); several of the traditional subtypes with eponyms are now re-named; additional subtypes have been added to include the laryngo-onycho-cutaneous syndrome (LOC syndrome; Shabbir syndrome); additional modes of transmission have been noted; and detailed summaries are available which describe transmission electron microscopy findings, abnormalities in basement membrane antigen expression, the most common types of gene mutations within each subtype, and comparative clinical findings.
References
Fine JD, Bauer EA, McGuire J, Moshell A, eds. Epidermolysis Bullosa: Clinical, Epidemiologic, and Laboratory Advances, and the Findings of the National Epidermolysis Bullosa Registry. Baltimore MD: Johns Hopkins University Press; 1999:520.
Fine JD, Hintner H, eds. Life with Epidermolysis Bullosa. Etiology, Diagnosis, Multidisciplinary Care and Therapy. New York NY: Springer; 2009:338.
Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol. 2008;58(6):931-950.
Fine JD, Bruckner-Tuderman L, Eady RA, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014;70(6):1003-11026.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367-384; quiz 385-386.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. J Am Acad Dermatol. 2009;61(3):387-402; quiz 403-404.
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
d) Type IV collagen
The tissue-bound autoantibodies directly beneath the lamina densa, co-localizing with the anchoring fibrils is depicted in this direct immunoelectron micrograph (Figure). The patient has either epidermolysis bullosa acquisita or the bullous eruption of systemic lupus erythematosus. Note: the faint gray linear band visible in the region of the dermoepidermal junction is the lamina densa “B”, which is devoid of overlying autoantibodies. The latter (“D”) are present solely within the sublamina densa zone. The only other autoimmune bullous disease which may have immunoglobulin deposits in linear array within the uppermost papillary dermis is linear IgA dermatosis (LAD), although most patients with LAD have their deposits within the lamina lucida; it is as yet unknown what is the antigenic target of these LAD-associated sublamina densa immunoreactants.
References
Gammon WR, Briggaman RA. Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Dermatol Clin. 1993;11(3):535-547.
Remington J, Chen M, Burnett J, Woodley DT. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Curr Dir Autoimmun. 2008;10:195-205.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
b) Generalized junctional epidermolysis bullosa, non-Herlitz subtype (JEB-nH)
c) EB simplex, Dowling-Meara subtype
d) Dominant dystrophic EB
e) Severe generalized recessive dystrophic EB (RDEB, severe generalized; formerly named RDEB, Hallopeau-Siemens)
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
a) Type IV collagen
b) Bullous pemphigoid antigen 1 (230-kD)
c) Laminin-332
d) Type VII collagen
e) Transglutaminase
,
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
b) Generalized junctional epidermolysis bullosa, non-Herlitz subtype (JEB-nH)
c) EB simplex, Dowling-Meara subtype
d) Dominant dystrophic EB
e) Severe generalized recessive dystrophic EB (RDEB, severe generalized; formerly named RDEB, Hallopeau-Siemens)
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
a) Type IV collagen
b) Bullous pemphigoid antigen 1 (230-kD)
c) Laminin-332
d) Type VII collagen
e) Transglutaminase
To learn the answers, go to page 2
{{pagebreak}}
BOARD REVIEW ANSWERS:
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
This child has symmetrically arrayed, periorificial, exuberant granulation tissue, a finding that is virtually pathognomonic of the Herlitz subtype of generalized JEB. Other skin sites affected by such a finding include the nape of the neck, lumbosacral region, and periungual folds. The nares and upper airway may also harbor granulation tissue like this, and may markedly obstruct air flow. JEB-H is an autosomal recessive disorder resulting from mutations arising in the genes encoding for any of the 3 subunits of laminin-332 (formerly named laminin-5), a 3-chained, cross-configured macromolecule which spans the laimina lucida of the dermoepidermal junction, attaching epidermis to dermis.
The classification system for inherited EB was revised in 2007 and published in the Journal of the American Academy of Dermatology in 2008. A significantly revised version incorporating several new subtypes characterized by novel phenotypes or newly discovered genes was published in the Journal of the American Academy of Dermatology in 2014. The key features of the revised classification system include that inherited EB has been expanded into 4 major groups, to now include Kindler syndrome; EB simplex has been subdivided into 2 major subgroups (basal and suprabasal); several of the traditional subtypes with eponyms are now re-named; additional subtypes have been added to include the laryngo-onycho-cutaneous syndrome (LOC syndrome; Shabbir syndrome); additional modes of transmission have been noted; and detailed summaries are available which describe transmission electron microscopy findings, abnormalities in basement membrane antigen expression, the most common types of gene mutations within each subtype, and comparative clinical findings.
References
Fine JD, Bauer EA, McGuire J, Moshell A, eds. Epidermolysis Bullosa: Clinical, Epidemiologic, and Laboratory Advances, and the Findings of the National Epidermolysis Bullosa Registry. Baltimore MD: Johns Hopkins University Press; 1999:520.
Fine JD, Hintner H, eds. Life with Epidermolysis Bullosa. Etiology, Diagnosis, Multidisciplinary Care and Therapy. New York NY: Springer; 2009:338.
Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol. 2008;58(6):931-950.
Fine JD, Bruckner-Tuderman L, Eady RA, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014;70(6):1003-11026.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367-384; quiz 385-386.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. J Am Acad Dermatol. 2009;61(3):387-402; quiz 403-404.
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
d) Type IV collagen
The tissue-bound autoantibodies directly beneath the lamina densa, co-localizing with the anchoring fibrils is depicted in this direct immunoelectron micrograph (Figure). The patient has either epidermolysis bullosa acquisita or the bullous eruption of systemic lupus erythematosus. Note: the faint gray linear band visible in the region of the dermoepidermal junction is the lamina densa “B”, which is devoid of overlying autoantibodies. The latter (“D”) are present solely within the sublamina densa zone. The only other autoimmune bullous disease which may have immunoglobulin deposits in linear array within the uppermost papillary dermis is linear IgA dermatosis (LAD), although most patients with LAD have their deposits within the lamina lucida; it is as yet unknown what is the antigenic target of these LAD-associated sublamina densa immunoreactants.
References
Gammon WR, Briggaman RA. Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Dermatol Clin. 1993;11(3):535-547.
Remington J, Chen M, Burnett J, Woodley DT. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Curr Dir Autoimmun. 2008;10:195-205.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
b) Generalized junctional epidermolysis bullosa, non-Herlitz subtype (JEB-nH)
c) EB simplex, Dowling-Meara subtype
d) Dominant dystrophic EB
e) Severe generalized recessive dystrophic EB (RDEB, severe generalized; formerly named RDEB, Hallopeau-Siemens)
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
a) Type IV collagen
b) Bullous pemphigoid antigen 1 (230-kD)
c) Laminin-332
d) Type VII collagen
e) Transglutaminase
BOARD REVIEW ANSWERS:
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
This child has symmetrically arrayed, periorificial, exuberant granulation tissue, a finding that is virtually pathognomonic of the Herlitz subtype of generalized JEB. Other skin sites affected by such a finding include the nape of the neck, lumbosacral region, and periungual folds. The nares and upper airway may also harbor granulation tissue like this, and may markedly obstruct air flow. JEB-H is an autosomal recessive disorder resulting from mutations arising in the genes encoding for any of the 3 subunits of laminin-332 (formerly named laminin-5), a 3-chained, cross-configured macromolecule which spans the laimina lucida of the dermoepidermal junction, attaching epidermis to dermis.
The classification system for inherited EB was revised in 2007 and published in the Journal of the American Academy of Dermatology in 2008. A significantly revised version incorporating several new subtypes characterized by novel phenotypes or newly discovered genes was published in the Journal of the American Academy of Dermatology in 2014. The key features of the revised classification system include that inherited EB has been expanded into 4 major groups, to now include Kindler syndrome; EB simplex has been subdivided into 2 major subgroups (basal and suprabasal); several of the traditional subtypes with eponyms are now re-named; additional subtypes have been added to include the laryngo-onycho-cutaneous syndrome (LOC syndrome; Shabbir syndrome); additional modes of transmission have been noted; and detailed summaries are available which describe transmission electron microscopy findings, abnormalities in basement membrane antigen expression, the most common types of gene mutations within each subtype, and comparative clinical findings.
References
Fine JD, Bauer EA, McGuire J, Moshell A, eds. Epidermolysis Bullosa: Clinical, Epidemiologic, and Laboratory Advances, and the Findings of the National Epidermolysis Bullosa Registry. Baltimore MD: Johns Hopkins University Press; 1999:520.
Fine JD, Hintner H, eds. Life with Epidermolysis Bullosa. Etiology, Diagnosis, Multidisciplinary Care and Therapy. New York NY: Springer; 2009:338.
Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol. 2008;58(6):931-950.
Fine JD, Bruckner-Tuderman L, Eady RA, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014;70(6):1003-11026.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367-384; quiz 385-386.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. J Am Acad Dermatol. 2009;61(3):387-402; quiz 403-404.
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
d) Type IV collagen
The tissue-bound autoantibodies directly beneath the lamina densa, co-localizing with the anchoring fibrils is depicted in this direct immunoelectron micrograph (Figure). The patient has either epidermolysis bullosa acquisita or the bullous eruption of systemic lupus erythematosus. Note: the faint gray linear band visible in the region of the dermoepidermal junction is the lamina densa “B”, which is devoid of overlying autoantibodies. The latter (“D”) are present solely within the sublamina densa zone. The only other autoimmune bullous disease which may have immunoglobulin deposits in linear array within the uppermost papillary dermis is linear IgA dermatosis (LAD), although most patients with LAD have their deposits within the lamina lucida; it is as yet unknown what is the antigenic target of these LAD-associated sublamina densa immunoreactants.
References
Gammon WR, Briggaman RA. Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Dermatol Clin. 1993;11(3):535-547.
Remington J, Chen M, Burnett J, Woodley DT. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Curr Dir Autoimmun. 2008;10:195-205.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
BOARD REVIEW ANSWERS:
1. This inherited blistering disease is:
a) Generalized junctional epidermolysis bullosa, Herlitz subtype (JEB-H)
This child has symmetrically arrayed, periorificial, exuberant granulation tissue, a finding that is virtually pathognomonic of the Herlitz subtype of generalized JEB. Other skin sites affected by such a finding include the nape of the neck, lumbosacral region, and periungual folds. The nares and upper airway may also harbor granulation tissue like this, and may markedly obstruct air flow. JEB-H is an autosomal recessive disorder resulting from mutations arising in the genes encoding for any of the 3 subunits of laminin-332 (formerly named laminin-5), a 3-chained, cross-configured macromolecule which spans the laimina lucida of the dermoepidermal junction, attaching epidermis to dermis.
The classification system for inherited EB was revised in 2007 and published in the Journal of the American Academy of Dermatology in 2008. A significantly revised version incorporating several new subtypes characterized by novel phenotypes or newly discovered genes was published in the Journal of the American Academy of Dermatology in 2014. The key features of the revised classification system include that inherited EB has been expanded into 4 major groups, to now include Kindler syndrome; EB simplex has been subdivided into 2 major subgroups (basal and suprabasal); several of the traditional subtypes with eponyms are now re-named; additional subtypes have been added to include the laryngo-onycho-cutaneous syndrome (LOC syndrome; Shabbir syndrome); additional modes of transmission have been noted; and detailed summaries are available which describe transmission electron microscopy findings, abnormalities in basement membrane antigen expression, the most common types of gene mutations within each subtype, and comparative clinical findings.
References
Fine JD, Bauer EA, McGuire J, Moshell A, eds. Epidermolysis Bullosa: Clinical, Epidemiologic, and Laboratory Advances, and the Findings of the National Epidermolysis Bullosa Registry. Baltimore MD: Johns Hopkins University Press; 1999:520.
Fine JD, Hintner H, eds. Life with Epidermolysis Bullosa. Etiology, Diagnosis, Multidisciplinary Care and Therapy. New York NY: Springer; 2009:338.
Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol. 2008;58(6):931-950.
Fine JD, Bruckner-Tuderman L, Eady RA, et al. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014;70(6):1003-11026.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367-384; quiz 385-386.
Fine JD, Mellerio J. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. J Am Acad Dermatol. 2009;61(3):387-402; quiz 403-404.
2. This patient has a blistering disease characterized by the presence of circulating and tissue-bound autoantibodies to:
d) Type IV collagen
The tissue-bound autoantibodies directly beneath the lamina densa, co-localizing with the anchoring fibrils is depicted in this direct immunoelectron micrograph (Figure). The patient has either epidermolysis bullosa acquisita or the bullous eruption of systemic lupus erythematosus. Note: the faint gray linear band visible in the region of the dermoepidermal junction is the lamina densa “B”, which is devoid of overlying autoantibodies. The latter (“D”) are present solely within the sublamina densa zone. The only other autoimmune bullous disease which may have immunoglobulin deposits in linear array within the uppermost papillary dermis is linear IgA dermatosis (LAD), although most patients with LAD have their deposits within the lamina lucida; it is as yet unknown what is the antigenic target of these LAD-associated sublamina densa immunoreactants.
References
Gammon WR, Briggaman RA. Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Dermatol Clin. 1993;11(3):535-547.
Remington J, Chen M, Burnett J, Woodley DT. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Curr Dir Autoimmun. 2008;10:195-205.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
